[Clinical analysis of 10 cases of multi-center tumor necrosis factor receptor-associated periodic syndrome].

To summarize the clinical characteristics of tumour necrosis factor receptor-associated periodic syndrome (TRAPS) in children. The clinical manifestations, laboratory tests, genetic testing and follow-up of 10 children with TRAPS from May 2011 to May 2021 in 6 hospitals in China were retrospectively analyzed. Among the 10 patients with TRAPS, including 8 boys and 2 girls.

[Clinical research of multisystem inflammatory syndrome in children].

To analyze the clinical characteristics of children with multisystem inflammatory syndrome (MIS-C) associated with SARS-CoV-2 in China, and to improve the understanding of MIS-C among pediatricians. Case series study.Collect the clinical characteristics, auxiliary examinations, treatment decisions, and prognosis of 64 patients with MIS-C from 9 hospitals in China from December 2022 to June 2023.

Evaluating the Efficacy of Amniotic Membrane in Treating Neonatal Extravasation.

Objective: Extravasation is leakage of material from a peripheral venous access into adjacent tissue, which results in tissue damage ranging from local irritation to necrosis and scar formation. Neonates are at extravasation risk with IV treatment because of their small, fragile veins and the long treatment period required. In this report, investigators assessed the efficacy of amniotic membrane (AM) as a biological dressing to heal extravasation wounds in neonates.

Validity of the Adrogué-Madias Formula for the Management of Acute Dysnatremias in Critically Ill Children: A Prospective Multicenter Analysis.

Objective: Current conventional formulas do not predict the expected changes in serum sodium after administration of various fluids to correct serum sodium abnormalities. The Adrogué-Madias formula is currently the preferred and widely used fluid prescription for adult patients with dysnatremias, but its therapeutic efficacy has not been validated in pediatric patients.

Methods: In this prospective study, we used the Adrogué-Madias formula for calculating the appropriate rate of various fluids administration to correct serum sodium abnormalities in 7 critically ill children with acute dysnatremias.

The characteristics of foreign bodies aspirated by children across different continents: A comparative review.

Background: Foreign body aspiration (FBA) is a common issue in pediatric emergencies, with regional variations. Various cultures and foods, parents' and physicians' inadequate experience, and lack of bronchoscopy equipment are some attributable factors in the regional variation of FBA.

Aim: To more accurately represent the demographic characteristics of aspirated foreign bodies (FBs) across various continents, this review attempted to provide organized information based on the reviewed articles.

Epidemiology of Pediatric Hand Injury at a Pediatric Department in Japan.

Objective: The hands and fingers are frequently injured among children. Therefore, this study aimed to describe the epidemiological characteristics of hand and finger injuries among Japanese children and identify preventive strategies.

Methods: This was a retrospective review of data of pediatric patients who visited the Kitakyushu City Yahata Hospital in Japan, between April 2018 and March 2019.

Clinical, laboratory data and outcomes of 17 Iranian citrullinemia type 1 patients: Identification of five novel gene mutations.

Citrullinemia type 1 is an autosomal recessive metabolic disease caused by gene mutations encoding argininosuccinic acid synthetase enzyme which is within the pathway of arginine and nitric oxide biosynthesis. Disease confirmation was done by gene mutation analysis using next-generation sequencing, DNA Sanger sequencing. The study group was 17 citrullinemia type 1 patients from 10 unrelated families referred to Iranian National Society for Study on Inborn Errors of Metabolism's clinic between 2008 and 2020.

Eculizumab exposure in children and young adults: indications, practice patterns, and outcomes-a Pediatric Nephrology Research Consortium study.

Background: Eculizumab is approved for the treatment of atypical hemolytic uremic syndrome (aHUS). Its use off-label is frequently reported. The aim of this study was to describe the broader use and outcomes of a cohort of pediatric patients exposed to eculizumab.

Diagnostic and prognostic value of T-wave amplitude difference between supine and orthostatic electrocardiogram in children and adolescents with postural orthostatic tachycardia syndrome.

Objective: To investigate the diagnostic and prognostic value of T-wave amplitude difference between supine and orthostatic electrocardiogram (ECG) in children and adolescents with postural orthostatic tachycardia syndrome (POTS).

Methods: A total of 100 children and adolescents (POTS group, 50 males and 50 females, aged at 11.0 ± 2.

Tau Is Elevated in Pediatric Patients on Extracorporeal Membrane Oxygenation.

Neurologic injury is a known and feared complication of extracorporeal membrane oxygenation (ECMO). Neurologic biomarkers may have a role in assisting in early identification of such. Axonal biomarker tau has not been investigated in the pediatric ECMO population.

Simultaneous detection of multiple inherited metabolic diseases using GC-MS urinary metabolomics by chemometrics multi-class classification strategies.

Metabonomics has been widely used in disease diagnosis and clinically practical methods often require the detection of multi-class bio-samples. In this work, multi-class classification methods were investigated to simultaneously discriminate among 6 inherited metabolic diseases (IMDs) and the normal instances using gas chromatography-mass spectrometry (GC-MS) of urine samples. Two common multi-class classification strategies, one-against-all (OAA) and one-against-one (OAO) were compared and enhanced using a novel ensemble classification strategy (ECS), which developed a set of sequential sub-classifiers by fusion of OAA and OAO and made the final classification decisions using softmax function.

Clinical, Radiologic, Pathologic, and Molecular Characteristics of Long-Term Survivors of Diffuse Intrinsic Pontine Glioma (DIPG): A Collaborative Report From the International and European Society for Pediatric Oncology DIPG Registries.

Purpose Diffuse intrinsic pontine glioma (DIPG) is a brainstem malignancy with a median survival of < 1 year. The International and European Society for Pediatric Oncology DIPG Registries collaborated to compare clinical, radiologic, and histomolecular characteristics between short-term survivors (STSs) and long-term survivors (LTSs). Materials and Methods Data abstracted from registry databases included patients from North America, Australia, Germany, Austria, Switzerland, the Netherlands, Italy, France, the United Kingdom, and Croatia.

Antimicrobial Stewardship Program Using Plan-Do-Study-Act Cycles to Reduce Unjustified Antibiotic Prescribing in Children Admitted With an Asthma Exacerbation.

Objective: Antimicrobial stewardship programs (ASPs) ensure appropriate antibiotic use, reduce health care costs, and minimize antibiotic resistance. National asthma guidelines do not recommend antibiotics during an exacerbation unless the child has an infection or comorbidities. The American Academy of Pediatrics (AAP) established a benchmark for unjustified antibiotic use at 6.

Lipoic acid metabolism and mitochondrial redox regulation.

Lipoic acid is an essential cofactor for mitochondrial metabolism and is synthesized using intermediates from mitochondrial fatty-acid synthesis type II, -adenosylmethionine and iron-sulfur clusters. This cofactor is required for catalysis by multiple mitochondrial 2-ketoacid dehydrogenase complexes, including pyruvate dehydrogenase, α-ketoglutarate dehydrogenase, and branched-chain ketoacid dehydrogenase. Lipoic acid also plays a critical role in stabilizing and regulating these multienzyme complexes.

The NQO1 bioactivatable drug, β-lapachone, alters the redox state of NQO1+ pancreatic cancer cells, causing perturbation in central carbon metabolism.

Many cancer treatments, such as those for managing recalcitrant tumors like pancreatic ductal adenocarcinoma, cause off-target toxicities in normal, healthy tissue, highlighting the need for more tumor-selective chemotherapies. β-Lapachone is bioactivated by NAD(P)H:quinone oxidoreductase 1 (NQO1). This enzyme exhibits elevated expression in most solid cancers and therefore is a potential cancer-specific target.

Lipid sensing by mTOR complexes via synthesis of phosphatidic acid.

mTOR, the mammalian target of rapamycin, integrates growth factor and nutrient signals to promote a transformation from catabolic to anabolic metabolism, cell growth, and cell cycle progression. Phosphatidic acid (PA) interacts with the FK506-binding protein-12-rapamycin-binding (FRB) domain of mTOR, which stabilizes both mTOR complexes: mTORC1 and mTORC2. We report here that mTORC1 and mTORC2 are activated in response to exogenously supplied fatty acids via the synthesis of PA, a central metabolite for membrane phospholipid biosynthesis.

Inter-center variation in death or tracheostomy placement in infants with severe bronchopulmonary dysplasia.

Objective: To estimate the presence and sources of inter-center variation (ICV) in the risk of death or tracheostomy placement (D/T) among infants with severe bronchopulmonary dysplasia (sBPD)Study design:We analyzed the Children's Hospitals Neonatal Database between 2010 and 2013 to identify referred infants born <32 weeks' gestation with sBPD. The association between center and the primary outcome of D/T was analyzed by multivariable modeling. Hypothesized diagnoses/practices were included to determine if these explained any observed ICV in D/T.

Glutathione Depletion, Pentose Phosphate Pathway Activation, and Hemolysis in Erythrocytes Protecting Cancer Cells from Vitamin C-induced Oxidative Stress.

The discovery that oxidized vitamin C, dehydroascorbate (DHA), can induce oxidative stress and cell death in cancer cells has rekindled interest in the use of high dose vitamin C (VC) as a cancer therapy. However, high dose VC has shown limited efficacy in clinical trials, possibly due to the decreased bioavailability of oral VC. Because human erythrocytes express high levels of Glut1, take up DHA, and reduce it to VC, we tested how erythrocytes might impact high dose VC therapies.

Extended-interval gentamicin administration in neonates: a simplified approach.

Objective: Gentamicin dosing is highly variable and remains complicated in the neonatal population. Traditional dosing in our unit resulted in an excessive number of elevated trough serum gentamicin levels. We hypothesized that one uniform gentamicin dose for neonates of all gestational ages will reduce the incidence of elevated trough levels from 50 to 10%.

Application of mid-frequency ventilation in an animal model of lung injury: a pilot study.

Background: Mid-frequency ventilation (MFV) is a mode of pressure control ventilation based on an optimal targeting scheme that maximizes alveolar ventilation and minimizes tidal volume (VT). This study was designed to compare the effects of conventional mechanical ventilation using a lung-protective strategy with MFV in a porcine model of lung injury. Our hypothesis was that MFV can maximize ventilation at higher frequencies without adverse consequences.

Autologous buccal mucosa graft augmentation for foreshortened vagina.

Background: Vaginal foreshortening after pelvic surgery or radiotherapy may lead to dyspareunia and decreased quality of life. Unfortunately, little literature exists regarding treatment options for this debilitating problem. Autologous buccal mucosal grafting has been previously reported for creation of a total neovagina and the repair of postvaginoplasty vaginal stenosis.

Ewing sarcoma of the scrotum.

Nonosseous Ewing sarcoma commonly occurs in the extremities or deep soft tissues. However, cutaneous and subcutaneous locations have been reported. A 3-year-old boy presented with a 2-year history of a painless, slowly growing mid-scrotal mass.

Predicting death or tracheostomy placement in infants with severe bronchopulmonary dysplasia.

Objective: To estimate the risk of death or tracheostomy placement (D/T) in infants with severe bronchopulmonary dysplasia (sBPD) born < 32 weeks' gestation referred to regional neonatal intensive care units.

Study Design: We conducted a retrospective cohort study in infants born < 32 weeks' gestation with sBPD in 2010-2011, using the Children's Hospital Neonatal Database. sBPD was defined as the need for FiO2 ⩾ 0.

Simultaneous steady-state and dynamic 13C NMR can differentiate alternative routes of pyruvate metabolism in living cancer cells.

Metabolic reprogramming facilitates cancer cell growth, so quantitative metabolic flux measurements could produce useful biomarkers. However, current methods to analyze flux in vivo provide either a steady-state overview of relative activities (infusion of (13)C and analysis of extracted metabolites) or a dynamic view of a few reactions (hyperpolarized (13)C spectroscopy). Moreover, although hyperpolarization has successfully quantified pyruvate-lactate exchanges, its ability to assess mitochondrial pyruvate metabolism is unproven in cancer.